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The clinical and pathological findings are described in a six-month-old female with an unusual congenital malformation, atresia of the orifice and hypoplasia of the left main coronary artery. The literature is reviewed and the clinical findings are discussed. A comparison has been made with the anatomic findings in true single coronary artery and with the angiographic findings in anomalous origin of the left coronary artery from the main pulmonary artery.
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